Treatment
10
Treatment of Nonclassic Congenital Adrenal Hyperplasia
➤ In children and adolescents with inappropriately early onset and rapid
progression of pubarche or bone age and in adolescent patients with overt
virilization, ES suggests glucocorticoid treatment of nonclassic congenital
adrenal hyperplasia. (2|⊕⊕
)
Technical remark: Risks and benefits of glucocorticoid therapy should be considered and
discussed with the patient's family.
➤ In asymptomatic nonpregnant individuals with nonclassic congenital
adrenal hyperplasia, ES recommends against glucocorticoid treatment.
(1|⊕⊕⊕
)
➤ In previously treated patients with nonclassic congenital adrenal
hyperplasia, ES suggests giving the option of discontinuing therapy when
adult height is attained or other symptoms resolve. (2|⊕⊕⊕
)
➤ In adult women with nonclassic congenital adrenal hyperplasia who also
have patient-important hyperandrogenism or infertility, ES suggests
glucocorticoid treatment. (2|⊕⊕
)
➤ In most adult males with nonclassic congenital adrenal hyperplasia,
ES suggests that clinicians generally not prescribe daily glucocorticoid
therapy. (2|⊕
)
Technical remark: Exceptions include infertility, testicular adrenal rest tumors or
adrenal tumors, and phenotypes that are intermediate between classic and nonclassic
phenotypes.
➤ In patients with nonclassic congenital adrenal hyperplasia, ES suggests
hydrocortisone stress dosing for major surgery, trauma, or childbirth
only if a patient has a suboptimal (<14 to 18 μg/dL, <400 to 500 nmol/L)
cortisol response to cosyntropin or iatrogenic adrenal suppression.
(2|⊕
)
Technical remark: A range is given for cortisol cut points due to greater specificity of
newer cortisol assays.
